Abstract: Objectives　To investigate the clinical characteristics of children with anti N-methy-D-aspartate receptor (NMDAR) encephalitis. Methods　The clinical data from 6 children with anti-NMDAR encephalitis were retrospectively analyzed. The related literatures of anti-NMDAR encephalitis were reviewed. Results　The age of 6 children with anti-NMDAR encephalitis were from 2 to 10 years old, 5 were males and 1 was female. Three children had prodromal symptoms. All 6 children had mental and behavior abnormalities and convulsions, 2 cases of ataxia, 2 cases of extrapyramidal symptoms, 2 cases of orofacial dyskinesia, and 3 cases of dysautonomia. There was no abnormality of routine examination of cerebrospinal fluid in all 6 children. The oligoclonal protein antibody in serum and cerebrospinal fluid were all positive in 5 children, and negative in serum and positive in cerebrospinal fluid in one child. The anti-NMDAR in serum and cerebrospinal fluid were positive in all 6 children. Four children had nonspecific abnormal brain MRI. EEG showed diffuse slow wave, and laced with epileptic form discharges in all 6 children. Non-convulsive status epilepticus was monitored in one child. Tumors were not detected in all 6 children. After diagnosis, one child was lost follow up, 5 children were treated by methylprednisolone and gamma globulin, and 4 children received antiepileptic treatment. After 1 year follow-up, three children were with no obvious complications, and 2 children had complications. Conclusions　Children with anti-NMDAR encephalitis would be hinted by the clinical manifestations such as mental and behavioral abnormalities, seizures, dyskinesia, dysautonomia, etc. Detecting the NMDAR antibody in the serum and cerebrospinal fluid may help the diagnosis.